Pulmonary Vein Stenosis

Primary pulmonary vein stenosis, also called idiopathic pulmonary vein stenosis, occurs when one or more of the four pulmonary veins develop abnormally. This can occur either with or without another type of congenital heart disease or lung disease. Acquired pulmonary vein stenosis, also called post-repair pulmonary vein stenosis, can develop after a heart catheterization procedure or surgery.

If it is associated with another type of congenital heart disease, pulmonary vein stenosis might be diagnosed before birth using fetal echocardiography. It is usually diagnosed after birth. Symptoms may include fast or heavy breathing, tiring easily, feeding problems, pale or blue skin color, or fluid in the lungs. Your child’s doctor may order the following tests:

Pediatric Cardiac Imaging
Imaging scans like an echocardiogram, a CT scan or CT angiogram, or a cardiac MRI can help identify narrowed pulmonary veins and determine the severity of the narrowing. 

Pulmonary Flow Scan 
Also known as a lung scan, this test helps your child’s doctors understand how much blood is flowing to the right side versus the left side of the lungs. Your child will receive an injection that has trace amounts of radioactive molecules. Then a special type of camera takes pictures of the radioactive molecules inside the lungs.

Diagnostic Cardiac Catheterization
Your child will be under general anesthesia during this minimally invasive procedure. An interventional cardiologist inserts a small, flexible tube called a catheter into a blood vessel (usually in the groin) and guided to the heart. This allows doctors to take measurements and capture detailed images from inside the heart, sometimes by injecting contrast (dye) into the arteries. Depending on what your child’s interventional cardiologist finds, they may proceed with catheter-based treatment at the same time so your child doesn’t have to undergo a separate procedure.

Children with severe or recurrent pulmonary vein stenosis may need multiple interventions -- cardiac catheterization or surgery -- over time.

Interventional Cardiac Catheterization 
While your child is under general anesthesia, an interventional cardiologist inserts a catheter into a blood vessel (usually in the groin) and guides it to the heart. Using tiny tools passed through the catheter, the doctor can inflate a small balloon to widen the narrowed pulmonary vein and then deflate and remove the balloon. Doctors may also insert a stent -- a small metal mesh tube -- into the vein to keep it open. 

Heart Surgery
Open-heart surgery is usually reserved for children with pulmonary vein stenosis that recurs or who also have another congenital heart disease. Pediatric heart surgeons will enlarge the vessels coming back to the heart by cutting or patching them and/or redirecting blood flow. 

Medications
Medicines may help slow recurrent tissue growth that narrows pulmonary veins that were previously treated with catheterization or heart surgery. These medications require routine monitoring to ensure your child is taking an effective dose while minimizing side effects. We offer expertise in medication management and can coordinate with your child’s local cardiologist(s) to order lab tests and measure medication levels.

Expert Team Approach to Your Child’s Care
Our pediatric interventional cardiologists (doctors who specialize in diagnosing and treating heart disease with catheter-based procedures), heart surgeons, cardiologists, physician assistants, nurse practitioners, and nurses are specially trained to care specifically for babies and children with the most severe heart problems. Our dedicated team of pediatric cardiac anesthesiologists have advanced training to minimize risk and reduce procedure time in children with heart disease.

Innovative Technology and Advanced Facilities
Heart catheterizations are performed in a sterile procedure room, like an operating room, called a cardiac catheterization lab. Duke Children's Hospital & Health Center houses two pediatric cardiac catheterization labs and a specialized hybrid operating room for surgical procedures that also use catheterization techniques. We use advanced 3D imaging technology to guide catheter-based interventions, improve safety, and reduce the use of radiation and contrast dye in young patients.

Recognized for Excellence
Duke Children’s is one of the few hospitals in the U.S. verified as a Level I Children's Surgery Center by the American College of Surgeons. This Level I designation recognizes our commitment to providing the safest and highest-quality surgical care to our young patients.